Abstract: ＴＯｂｊｅｃｔｉｖｅ：To observe the clinical features, pathological presentation, and prognosis in antiglomerular basement membrane (GBM) nephritis patients with antineutrophil cytoplasmic antibody (ANCA) positive.
Ｍｅｔｈｏｄｏｌｏｇｙ：The patients diagnosed with antiGBM nephritis between January 2004 and December 2020 at the National Clinical Research Center of Kidney Diseases were selected to analyze the clinical and pathological characteristics and prognosis of patients with ANCA positive ， and compared with the cohort of antiGBM without ANCA.
Ｒｅｓｕｌｔｓ：Of the 192 antiGBM nephritis, 33 patients coexisted MPOANCA with 2 patients simultaneous with PR3ANCA. The elder onset age, female patient ratio, and serum albumin in the ANCA group were higher than those in the control group (P<005), but 24hr proteinuria and titer of antiGBM antibody were lower (P<005). There was no significant difference in the pathological features except for acute lesions of tubulointerstitial (P=0027). There was no significant difference in survival rate and renal survival at oneyear followup between the two groups.
Ｃｏｎｃｌｕｓｉｏｎ：AntiGBM nephritis with ANCA patients were similar to those without ANCA patients in the clinical and pathological characteristics, with poor prognosis.